A Case of Type 1 Congenital Pulmonary Airway Malformation (CPAM) in an Eight-Year-Old Girl Without a History of Recurrent Pneumonia or Respiratory Failure, Presenting With a Pulmonary Abscess.
Ryutaro Ohira, Natsuho Adachi, Shoichiro Kanda, Amane Yamamoto, Motoki Ebihara, Tomo Kakihara, Jun Fujishiro, Keiichi Takizawa
Abstract
Open AccessCongenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung, most often diagnosed in infancy or early childhood due to recurrent pneumonia or respiratory distress. We report the case of an eight-year-old girl who presented with fever, cough, hemoptysis, and chest pain, and was ultimately found to have pulmonary abscesses in the right upper and middle lobes. She had no prior history of recurrent respiratory infections or neonatal distress. Chest computed tomography revealed multilocular cystic lesions involving two lobes, raising suspicion for CPAM. The patient received prolonged intravenous and oral antibiotic therapy, followed by surgical resection consisting of a right upper lobectomy and wedge resection of the middle lobe. Histopathological examination confirmed Type 1 CPAM. She has remained free of recurrence since surgery. This case is notable for the unusually late presentation of Type 1 CPAM in school-aged children without prior symptoms, discovered only after the development of pulmonary abscesses. The absence of recurrent pneumonia or abnormal imaging made differentiation from a primary pulmonary abscess challenging. Given the risks of recurrent infections and malignant transformation, including mucinous adenocarcinoma and pleuropulmonary blastoma, surgical resection remains the preferred treatment, even in atypical or incidentally detected cases. This report underscores the importance of considering congenital cystic lung disease in the differential diagnosis of pediatric pulmonary abscess and highlights the role of thorough evaluation and timely surgical management in achieving favorable outcomes.